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path final > Haem: Lymphoma 2, CLL and Lymphoproliferative disorder > Flashcards

Haem: Lymphoma 2, CLL and Lymphoproliferative disorder Flashcards

1
Q

How does a lymphoma present clinically?

(regardless of type!)

A

Painless lymphadenopathy
* commonly neck, axilla, groin
* if intracavity lymph node, then cannot be palpated but obstructive symptoms can develop!
* pain after alcohol = hodgkin’s (does not occur in NHL)

Organ infiltration
* Ocular lymphoma
* skin nodules - mucosis fungoides

Recurrent infections
* lymphocytes are ineffective therefore poor immune reaction

FLAWS
* B symptoms in high grade aggressive lymphomas due to increased metabolic state.

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2
Q

What obstructive symptoms can arise from a lymphoma causing an enlarged intra-cavity lymph node

A

Ureter obstruction –> renal failure
Bile duct obstruction –> obstructive jaundice
IVC / SVC —> oedema
Tracheal obstruction –> respiratory distress

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3
Q

What are the important investigations to carry out when diagnosing a lymphoma

A

Histology: biopsy + immunophenotype + molecular tools
- Crucial for dx of type of lymphoma + prognosis

Anatomical Staging: PET-CT + Bone marrow biopsy (if bone marrow could be affected) + Lumbar puncture (if CNS involvement likely)

Bloods:
* LDH - marker of cell turnover
* Albumin - important for liver function
* HIV serology - HIV can predispose to NHL (HTLV1 serology may also be important)
* Hepatitis B serology - Lymphoma treatment may deplete B cells resulting in fulminant liver failure due to reactivation of hepatitis B in chronic carriers

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4
Q

Demographics of a typical patient with Hodgkin’s Lymphoma

A

Bimodal age distribution
- 20-29 (commonly female = nodular sclerosing HL)
- >60 (commonly male)

Overall commonly male

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5
Q

Describe the typical presentation of Hodgkin’s lymphoma.

A

Painless lymphadenopathy
* With contigous spread.
* Pain on alcohol
* nodes tend to mediastinal/cervical!

Compression symptoms

B symptoms

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6
Q

Outline the staging system used for Hodgkin’s Lymphoma

A

Done using PET-CT scan - allows to see highly active lymph nodes (note that kidneys and bladder will be lit up due to excretion not!)

Ann Arbor Staging
Stage 1 - one LN region (Ln region can include spleen!)
Stage 2 - two or more LN regions same side of diaphragm
Stage 3 - two or more LN regions both sides of diaphragm
Stage 4 - involvement of extra nodal sites (Liver, BM)
+
A: no constitutional symptoms
B: constitutional symptoms

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7
Q

Outline the management for Hodgkin’s Lymphoma

A
  1. Combination therapy (ABVD) 2-6 cycles. After 2nd cycle a PET-CT is done to check treatment efficacy (depending on it do more or less)
  2. Radiotherapy: may be used alongside chemo in bulky areas but VERY HIGH RISK OF BREAST CANCER!

For relapsed patients: second line chemo agents or stem cell transplant.

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8
Q

What are the two most common types of non-Hodgkin lymphoma?

A

Diffuse large B cell lymphoma (DLBCL) (30-40% of all NHL)

Follicular lymphoma (35% of all NHL)

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9
Q

List some types of non-Hodgkin lymphoma that are:

  1. Very agressive
  2. Aggresive
  3. Indolent
A
  1. Very agressive
    • Burkitt’s lymphoma
    • T or B cell lymphoblastic lymphoma/leukaemia
  2. Aggressive
    • Diffuse large B cell lymphoma
    • Mantle cell lymphoma
  3. Indolent
    • Follicular lymphoma
    • Small lymphocytic lymphoma (CLL)
    • MALToma
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10
Q

What is the correlation between how aggressive a lymphoma is and how curable it is?

A

The more aggressive it is, the more curable

Indolent lymphoma can enter remission but is more likely to recur

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11
Q

Which factors are taken into account by the international prognostic index (IPI) for lymphoma?

A
  • Age >60
  • High LDH
  • Performance status 2-4
  • Stage III or IV
  • More than one extranodal site
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12
Q

Outline the staging method used for NHL

A

Ann Arbor staging - the same as Hodgkin’s lymphoma

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13
Q

Broadly speaking, what are the treatment options to non-Hodgkin lymphomas?

A
  • Monitor only (in indolent lymphoma)
  • Urgent chemotherapy
  • Non-chemotherapy treatment (e.g. Antibiotic for H. Pylori)
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14
Q

Which chemotherapy treatment is usually used for diffuse large B cell lymphoma?

A
  • R-CHOP - 6-8 cycles
    • Rituximab + CHOP (chemo agents)

Achieves a 50% cure rate. Failure to achieve cure rate = Auto-SCT or CAR-T (T-cell therapy)

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15
Q

What treatment option may be considered for patients with diffuse large B cell lymphoma who relapse?

A

Autologous stem cell transplantation

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16
Q

What is the usual first-line treatment approach to follicular lymphoma? Outline the indications to escalate treatment

A

Watch and wait

Only treat it clinically indicated (e.g. compression symptoms, massive nodes, recurrent infection)

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17
Q

Which chemotherapy regimen may be used in the treatment of follicular lymphoma?

A
  • R-CVP
    • Rituximab
    • Cyclophosphamide
    • Vincristine
    • Prednisolone
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18
Q

Which lymphoid tissue tends to be affected by marginal zone lymphoma?

A

Extranodal lymphoid tissue (e.g. MALT)

19
Q

What is the cause of marginal zone lymphoma?

A
  • H. pylori infection - gastric MALToma
  • Sjogren’s syndrome - parotid lymphoma
  • Hashimoto’s thyroiditis - thyroid lymphoma
  • Psittaci infection - lacrimal gland
20
Q

Where is marginal zone lymphoma most commonly seen and how does it tend to present?

A
  • Usually in the stomach
  • Presenting with dyspepsia or epigastric pain
  • Usually Stage 1{E} (E=extranodal)
  • B symptoms are uncommon
21
Q

Outline the process of Gastric MALT lymphomagenesis.

A
  • Lymphocytes will respond to H. pylori infection and proliferate
  • At some point, they will over-proliferate and develop cancer-like features but they will still be dependent on antigenic stimulation by H. pylori
  • At this point, treating H. pylori will treat the lymphoma
22
Q

How might gastrict MALToma stage I-II disease be treated?

A
  • Triple therapy to eradicate H. pylori (2 antibiotics + 1 PPI)
  • Repeat breath test at 2 months
  • Repeat endoscopy every 6 months for 1-2 years then annually

NOTE: failure may require chemotherapy

23
Q

What are the main features of enteropathy-associated T cell lymphoma?

A
  • Mature T cells
  • Involves small intestines
  • Aggressive
  • Caused by chronic antigenic stimulation by gliadin/gluten
24
Q

Describe the typical presenting features of enteropathy-associated T cell lymphoma.

A
  • Abdominal pain/ obstruction/ bleeding/ perforation
  • Malabsorption
  • Systemic symptoms
25
Q

Why is it important to prevent EATL by following a strict gluten-free diet?

A

EATL responds poorly to chemotherapy and is usually fatal. Hence prevention is key.

26
Q

What is the most common leukaemia in the Western world?

A

Chronic lymphocytic leukaemia

27
Q

What are the typical laboratory findings in a patient with CLL?

A
  1. Lymphocytosis (commonest cause of lymphocytosis in elderly!)
  2. Smear cells
  3. Normocytic normochromic anaemia
  4. Thrombocytopaenia
  5. Bone marrow lymphocytic replacement of normal marrow elements

NOTE: it is indolent so is often only picked up on routine blood tests in asymptomatic patient.

28
Q

What distinctive antigen phenotype (presence and absence) is suggestive of:

  • Mature B cells
  • Mature T cells
A
  1. Mature B cells:
    • CD19 positive
    • CD5 negative
  2. Mature T cells:
    • CD19 negative
    • CD5 positive
    • CD3 positive
    • CD4 or CD8 positive
29
Q

Which antigen phenotype is suggestive of CLL?

A

CD5+ and CD23+ B cells (i.e. CD19+ and CD5+)

NOTE: this could potentially also be mantle cell lymphoma

30
Q

Which staging system is used for CLL?

A

Rai and Binet

Binet: stages A-C depending on number of lymphoid areas (< or > 3, Hb and platelets)

31
Q

Which laboratory tests are used in CLL to help gauge prognosis?

A

CD38 expression (associated with poor prognosis)

Cytogenetics (FISH)

Immunoglobulin gene mutation status (IgH mutated or unmutated)

32
Q

What are VH genes?

A

The genes that encode the ‘variable heavy’ chain and undergoes somatic hypermutation by VDJ recombination.

33
Q

What is the difference between the VH genes of pre- and post-germinal centre B cells?

A

Pre-germinal centre: VDJ section is unmutated and looks identical to germline

Post-germinal centre: undergone somatic hypermutation so VDJ is mutated and looks different to germline

34
Q

How does VH gene mutations affect prognosis?

A

Unmutated = poor prognosis

Mutated = better prognosis

35
Q

What is an important chromosomal abnormality in CLL that is tested for using FISH?

A
  • Deletion of 17p (Tp53)
  • This is part of the p53 tumours suppressor gene
  • This deletion is associated with a poor prognosis as it causes abscence of tumour suppressor gene
36
Q

Describe the immunoglobulin levels you would expect to see in CLL.

A

Hypogammaglobulinaemia

Because the malignant B cells are suppressing antibody production by other B cells

37
Q

What is the term used to describe CLL changing into a high grade lymphoma?

A

Richter transformation - 1% risk per year

38
Q

What are some supportive measures used in the treatment of CLL?

A
  • Vaccination (flu, pneumococcus)
  • Infection prophylaxis and treatment (may includ aciclovir, PCP prophylaxis, IVIG)
39
Q

How would autoimmune cytopaenias caused by CLL be treated?

A

Steroids

NOTE: 2nd line is rituximab

40
Q

How would a Richter transformation be treated?

A

R-CHOP

41
Q

What is leukaemia-directed therapy in patients with CLL ?

A
  • Usually involves watching and waiting
  • Patients with indicaitons for treatment:
    • chemotherapy OR direct target therapy (more common)!
42
Q

What are some indications for treatment of CLL?

A
  • Progressive lymphocytosis (more than doubling in <6 months)
  • Progressive bone marrow failure
  • Massive or progressive lymphadenopathy/splenomegaly
  • Systemic symptoms (B symptoms)
  • Autoimmune cytopaenias
43
Q

What are the direct target therapy options for patients with CLL?

A

Bruton Tyrosine Kinase Inhibitors - ibrutinib, idelalisib
* bruton tyrosine kinase allows destruction and suppression of B cells

Bcl2 Inhibitors - venetoclax
* targets and induces the Bcl2 apoptotic pathway

44
Q

Age of onset of Hodgkin’s, Diffuse B cell lymphoma and Burkitt’s lymphoma

A

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